Clear Cell Odontogenic Carcinoma Harboring the EWSR1ATF1 Fusion Gene: Report of a Rare Case

  • Thalita Santana
  • Felipe Ledo de Andrade
  • Maria Carolina de Sousa Melo
  • Glauber Bareia Liberato da Rocha
  • Marília TrierveilerEmail author
Case Reports


Clear cell odontogenic carcinoma (CCOC) is a rare and aggressive malignant epithelial neoplasm, which occurs most frequently in the mandible of elderly patients. Morphologically, CCOC shares similar characteristics with other clear cell tumors, especially hyalinizing clear cell carcinoma of the salivary glands (HCCC). Both CCOC and HCCC are known to harbor EWSR1 rearrangements, especially the EWSR1ATF1 gene fusion, which indicates a possible link between the two lesions. So far, this fusion has been demonstrated in five cases of CCOC in the literature. Herein, we add another CCOC case to the literature, which arose in the mandible of an 82-year-old female patient and was proven to harbor the EWSR1ATF1 gene fusion. Immunohistochemically, this case was focally positive for CK7, CK14, CK19 and p63. The patient was referred to surgical treatment; however, she died of disease 2 months after the diagnosis, thereby demonstrating the aggressive nature of this tumor.


Odontogenic tumors Clear cell odontogenic carcinoma Salivary gland neoplasms Hyalinizing clear cell carcinoma Malignant odontogenic tumor EWSR1ATF1 gene fusion 



This study was supported by Coordenação de Aperfeiçoamento de Pessoal de Nível Superior–Brasil (CAPES)–Finance Code 001. T. Santana received a scholarship from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) during the period in which this study was carried out.


This study was supported by Coordenação de Aperfeiçoamento de Pessoal de Nível Superior–Brasil (CAPES)–Finance Code 001. During the preparation of this manuscript, T. Santana received a scholarship from from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq).

Compliance with Ethical Standards

Conflict of interest

The authors state that there is no conflict of interest to disclose.


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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Oral and Maxillofacial Pathology, School of DentistryUniversity of São Paulo–USPSão PauloBrazil
  2. 2.Department of Oral and Maxillofacial SurgeryMandaqui Hospital ComplexSão PauloBrazil
  3. 3.Dentistry Division, Clinics Hospital of the Medical SchoolUniversity of São Paulo Paulo–USPSão PauloBrazil

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